| Phacomatosis Pigmentokeratotica: A Further Case without Extracutaneous Anomalies and Review of the Condition. | |
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MedLine Citation:
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PMID: 22082467 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Epidermal nevus syndrome is the term for the association of an epidermal nevus and extracutaneous anomalies, including neurologic, ophthalmic, and skeletal defects. Epidermal nevus syndromes include different disorders that share the feature of mosaicism. Phacomatosis pigmentokeratotica (PPK) is a distinctive new epidermal nevus syndrome first described in 1996 characterized by the presence of multiple organoid nevi with sebaceous differentiation, a speckled lentiginous nevus, and skeletal and neurologic abnormalities. Only a handful of cases of PPK without extracutaneous manifestations have been reported. We report here an individual with PPK with only cutaneous signs and confirm this distinctive syndrome has two subtypes according to the presence or absence of extracutaneous involvement. |
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Authors:
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Rattanavalai Chantorn; Tor Shwayder |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Pediatric dermatology Volume: 28 ISSN: 1525-1470 ISO Abbreviation: Pediatr Dermatol Publication Date: 2011 Nov |
Date Detail:
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Created Date: 2011-11-15 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8406799 Medline TA: Pediatr Dermatol Country: United States |
Other Details:
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Languages: eng Pagination: 715-9 Citation Subset: IM |
Copyright Information:
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© 2011 Wiley Periodicals, Inc. |
Affiliation:
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Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok Thailand Department of Dermatology, Henry Ford Hospital, Detroit, Michigan. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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